Adult-onset Still's disease presenting with acute liver failure caused by hemophagocytic lymphohistiocytosis

Hemophagocytic lymphohistiocytosis is a well-known complication of adult-onset Stills disease but an unusual cause for acute liver failure. Here we present a young female with the background of fever, arthritis, rash presenting with acute onset liver failure with hepatic encephalopathy and bleeding. The diagnostic evaluation was negative for infections, autoimmune disorders or malignancies and fulfilled the criteria for adult-onset Stills disease and hemophagocytic lymphohistiocytosis resulting in acute liver failure. Treatment with liver failure regime and immunomodulation ensued a quick and complete recovery

Lupus anticoagulant- hypoprothrombinemia syndrome detected in a patient with systemic lupus erythematosus with lupus nephritis

Systemic lupus erythematosus is well known to cause renal and haematological manifestations. Here we present a young female presenting with nephrotic syndrome fulfilling criteria for systemic lupus erythematosus found to have lupus anticoagulant hypoprothrombinemia syndrome on the evaluation for deranged coagulation profile. Prompt immunosuppressive therapy resulted in both renal and haematological resolution

Low-Dose Adrenaline, Promethazine, and Hydrocortisone in the Prevention of Acute Adverse Reactions to Antivenom following Snakebite: A Randomised, Double-Blind, Placebo-Controlled Trial

Background Envenoming from snakebites is most effectively treated by antivenom. However, the antivenom available in South Asian countries commonly causes acute allergic reactions, anaphylactic reactions being particularly serious. We investigated whether adrenaline, promethazine, and hydrocortisone prevent such reactions in secondary referral hospitals in Sri Lanka by conducting a randomised, double-blind placebo-controlled trial. Methods and Findings In total, 1,007 patients were randomized, using a 2×2×2 factorial design, in a double-blind, placebo-controlled trial of adrenaline (0.25 ml of a 1:1,000 solution subcutaneously), promethazine (25 mg intravenously), and hydrocortisone (200 mg intravenously), each alone and in all possible combinations. The interventions, or matching placebo, were given immediately before infusion of antivenom. Patients were monitored for mild, moderate, or severe adverse reactions for at least 96 h. The prespecified primary end point was the effect of the interventions on the incidence of severe reactions up to and including 48 h after antivenom administration. In total, 752 (75%) patients had acute reactions to antivenom: 9% mild, 48% moderate, and 43% severe; 89% of the reactions occurred within 1 h; and 40% of all patients were given rescue medication (adrenaline, promethazine, and hydrocortisone) during the first hour. Compared with placebo, adrenaline significantly reduced severe reactions to antivenom by 43% (95% CI 25–67) at 1 h and by 38% (95% CI 26–49) up to and including 48 h after antivenom administration; hydrocortisone and promethazine did not. Adding hydrocortisone negated the benefit of adrenaline. Conclusions Pretreatment with low-dose adrenaline was safe and reduced the risk of acute severe reactions to snake antivenom. This may be of particular importance in countries where adverse reactions to antivenom are common, although the need to improve the quality of available antivenom cannot be overemphasized